Multiple osteotomies and intramedullary nailing with neck cross-pinning for shepherd's crook deformity in polyostotic fibrous dysplasia: 7 femurs with a minimum of 2 years follow-up

Background?In polyostotic fibrous dysplasia, particularly in lesions of the proximal femur, pathological fracture and coxa vara deformity (including shepherd's crook deformity) are likely to develop and progress.

Patients and methods?In 7 femurs with shepherd's crook deformity (5 patients), we performed intramedullary nailing by using multiple osteotomies and two screws crossing the femoral neck.

Results?Restoration of the neck shaft angle of the femur was obtained from an average of 92° prior to surgery to 129° after surgery. During the minimum 2-year follow up, no loss of neck shaft angle of the femur and no refracture occurred. All patients were able to return to normal activities of daily living.

Interpretation?In polyostotic fibrous dysplasia, multiple osteotomies and intramedullary nailing with neck cross-pinning can be used to correct developed or progressing shepherd's crook deformity, and to prevent recurrence and refracture.     

Deformity correction by external fixation and/or intramedullary nailing in hypophosphatemic rickets

Background There are many modalities of treatment for complex lower extremity deformity in hypophosphatemic rickets. We evaluated the outcomes of deformity correction using an external fixation and/or intramedullary nailing in hypophosphatemic rickets

Patients and methods 55 segmental deformities (20 femora, 35 tibiae) from 20 patients were examined retrospectively. There were 9 children and 11 adults. Distraction osteogenesis was used in 28 segments and acute deformity correction in 27. External fixation was applied in 24 segments, intramedullary nailing in 6, and external fixation and intramedullary nailing in 25.

Results There were 18 major and 13 minor complications in 26 of 28 segments with distraction osteogenesis, and 13 major and 10 minor complications in 19 of 27 segments with acute correction. Recurrent deformity or refracture occurred in 10 of 21 segments with distraction osteogenesis by external fixation only, 4 of 6 with acute correction by intramedullary nailing, and 1 of 25 with distraction osteogenesis or acute correction by external fixation and intramedullary nailing. Nail-related complications occurred in 3 of 6 with intramedullary nailing and 2 of 25 with external fixation and intramedullary nailing.

Interpretation External fixation and intramedullary nailing can be recommended to prevent complications during or after deformity correction in hypophosphatemic rickets.     

Surgical treatment of femoral deformities in polyostotic fibrous dysplasia and McCune-Albright syndrome: A literature review

BACKGROUNDSurgical correction of femoral deformities in polyostotic fibrous dysplasia (PFD) or McCune-Albright syndrome (MAS), such as coxa vara or shepherd’s crook deformity, is a challenge. AIMTo evaluate the treatment of patients with femoral deformities caused by PDF or MAS treated by osteotomies and stabilized with different methods, by analyzing the most relevant studies on the topic. METHODSA literature search was performed in Medline database (PubMed). Articles were screened for patients affected by PFD or MAS surgically managed by osteotomies and stabilized with different methods. RESULTSThe initial search produced 184 studies, with 15 fulfilling the eligibility criteria of our study. Selected articles (1987-2019) included 111 patients overall (136 femurs). CONCLUSIONBased on our results, the preferred method to stabilize corrective osteotomies is intramedullary nailing with neck cross pinning. When the deformity is limited to the proximal part of the femur, a screw or blade plate may be used, although there is a high risk of fracture below the plate. When the femur is entirely involved, a two-stage procedure may be considered.     

Fibrous dysplasia of the proximal femur: surgical management options and outcomes

Purpose

The management of proximal femoral deformity in fibrous dysplasia (FD) is a challenge to the orthopaedic surgeon. The purpose of this study was to analyze the various presentations of FD of proximal femur and the results of the various treatment modalities for the same.

Methods

This is a retrospective cohort study of 23 patients (24 femora) with FD who underwent surgery for the proximal femur. The study sample included 14 males, nine females. Ten patients had a monostotic disease, eight patients had polyostotic disease, and five patients had McCune-Albright syndrome.

Results

Group 1: shepherd crook deformity—included five patients who underwent femoral neck osteotomy. Four patients had intramedullary (IM) nailing with neck cross-pinning and all patients showed union. One patient was stabilized with external fixation, which failed. Group 2: nine patients (ten femora) presented with frank pathological fracture. Nine underwent fixation with IM nailing, one with locking plate and screws. Three patients had to undergo more than one procedure and all fractures showed good union. Group 3: nine patients who presented with bone cyst and pain. All patients underwent biopsy; four of them had curettage with bone graft.

Conclusion

Shepherd crook deformity can be treated by a well-planned osteotomy and fixation with intramedullary implants with neck cross-pinning. Frank pathological fractures fixation with an intramedullary nail has excellent results even if not accompanied by resolution of the fibrodysplastic lesion. More than one procedure may be required. External fixation is not an optimal choice for fixation of femoral osteotomies in FD.     

股骨近端纤维结构不良的手术治疗

目的：通过对股骨近端纤维结构不良病例进行回顾性研究,总结肿瘤学和功能学结果,探讨其治疗方法及效果。方法：2007年4月至2009年1月,收治15例股骨近端纤维结构不良患者,男9例,女6例;年龄16～32岁,平均25岁;单侧病变12例,双侧病变3例;单骨型12例,多骨型3例;病程2个月～16年,平均2年。股骨近端纤维结构不良伴髋内翻2例,颈干角分别为80°和100°,股骨长度较对侧短缩分别为5cm和3cm。所有患者采用病灶刮除、打压植骨（同种异体人工骨和/或自体髂骨）、内固定治疗,其中2例牧羊拐畸形者采用外翻截骨矫形术。结果：所有患者获随访,时间12～32个月,2例股骨近端牧羊拐畸形患者经截骨矫形后畸形矫正,颈干角恢复,股骨长度延长分别为4cm和3cm,术后4个月扶双拐下地行走。所有患者术后病变无复发及内固定物松动,植骨区术后3个月可见局部骨吸收,术后8～12个月植骨区骨愈合,疼痛消失,步态正常。结论：彻底刮除病灶、植骨及有效内固定是治疗股骨近端纤维结构不良的有效方法,对伴有牧羊拐畸形者应同时行外翻截骨以恢复髋关节功能。     

An economic analysis of management strategies for closed and open grade I tibial shaft fractures

Background Closed and open grade I (low-energy) tibial shaft fractures are a common and costly event, and the optimal management for such injuries remains uncertain.

Methods We explored costs associated with treatment of low-energy tibial fractures with either casting, casting with therapeutic ultrasound, or intramedullary nailing (with and without reaming) by use of a decision tree.

Results From a governmental perspective, the mean associated costs were USD 3,400 for operative management by reamed intramedullary nailing, USD 5,000 for operative management by non-reamed intramedullary nailing, USD 5,000 for casting, and USD 5,300 for casting with therapeutic ultrasound. With respect to the financial burden to society, the mean associated costs were USD 12,500 for reamed intramedullary nailing, USD 13,300 for casting with therapeutic ultrasound, USD 15,600 for operative management by non-reamed intramedullary nailing, and USD 17,300 for casting alone.

Interpretation Our analysis suggests that, from an economic standpoint, reamed intramedullary nailing is the treatment of choice for closed and open grade I tibial shaft fractures. Considering financial burden to society, there is preliminary evidence that treatment of low-energy tibial fractures with therapeutic ultrasound and casting may also be an economically sound intervention.     

Multiple osteotomies with Zickel nail fixation for polyostotic fibrous dysplasia involving the proximal part of the femur

Involvement of the proximal part of the femur by polyostotic fibrous dysplasia is a progressive and disabling condition that leads to deformity. Morbidity is high in terms of recurrent fracture and deformity. Conventional methods of treatment have been ineffective in controlling these problems. The cases of four patients were reviewed to determine the natural history of polyostotic fibrous dysplasia involving the proximal part of the femur. In six femora, multiple osteotomies with fixation using a Zickel nail was utilized to provide definitive control of deformity and recurrent fracture and to allow the patients to return to normal activities.     

Treatment of femoral fractures associated with fibrous dysplasia: a case report

Fibrous dysplasia is a rare condition in which bone tissue is replaced by fibro-osseous lesions. A sixteen-year-old male patient with fibrous dysplasia underwent plate fixation for subtrochanteric fracture of the proximal femur. Eighteen months postoperatively, progressive femoral deformity and refracture occurred. Upon failure in three-month conservative treatment, the plate was removed and intramedullary fixation was performed. Control radiographs at the end of 18 months after intramedullary fixation showed complete fracture healing. This case emphasizes that intramedullary fixation should be the first choice to treat femoral fractures and to prevent refractures in patients with fibrous dysplasia.     

Orthopedic management in children with fibrous dysplasia of bone]

The method and results of treatment in 9 children with fibrous dysplasia of bone and 3 children with Albright syndrome are presented. Curettage and filling of the defect with autologous and/or lyophilized grafts failed in all 5 attempted cases. Due to pathological fracture or deformity Rush nailing combined with multilevel osteotomies has been performed within 5 femoral bones and 4 tibial bones. Healing was undisturbed, neither fracture nor deformity recurred. Valgus osteotomy has been done in 2 children with pseudoarthrosis of the femoral neck. In one case osteotomy healed, in another bony union is not completed yet but the neck-shaft angle remained unchanged. In authors' opinion curettage and grafting proved ineffective; Rush nailing rendered good results in both fracture and deformity treatment.     

Valgus osteotomy in combination with dynamic hip screw fixation for fibrous dysplasia with shepherd’s crook deformity

Background and objectives

The treatment of fibrous dysplasia with shepherd’s crook deformity is a big challenge. The purpose of this study was to investigate the clinical effect of valgus osteotomy in combination with dynamic hip screw (DHS) fixation to treat fibrous dysplasia with shepherd’s crook deformity.

Method

Twenty-one clinical cases of femoral fibrous dysplasia with shepherd’s crook deformity treated between April 2001 and May 2010 were retrospectively analyzed. The valgus osteotomy and internal fixation were performed for these patients. Six patients underwent DHS and trochanter stabilizing plate internal fixation, and the other 15 cases were stabilized by DHS fixation.

Results

Patients were followed for 19–128 months. The neck-shaft angle was corrected from 89° (range 65°–107°) preoperatively to 129° (range 119°–140°) postoperatively. Limb-length discrepancy was corrected from 3.0 (range 1.8–4.5) cm preoperatively to 0.7 (range 0–1.9) cm postoperatively. All osteotomies had healed at the final follow-up examination. The clinical scores, which were evaluated by the modified criteria of Guille, improved from an average of 2.9 (range 1–7) to 8.5 (range 6–10).

Conclusion

Our results demonstrate that valgus osteotomy in combination with DHS internal fixation is an easy and effective method for the treatment of fibrous dysplasia with shepherd’s crook deformity. It can restore the neck-shaft angle and re-establish the mechanical alignment of the femur to improve function.     

髓内钉固定治疗青少年股骨干骨折的疗效分析

目的:探讨髓内钉固定治疗青少年股骨干骨折的临床疗效。方法:回顾性分析2017年5月至2019年10月期间佛山市中医院小儿骨科收治的30例青少年股骨干骨折患者资料。男20例,女10例;年龄为12~17岁,平均14岁;左侧13例,右侧17例。手术均采用髓内钉固定：12例患者行切开复位固定,18例患者行闭合复位内固定。记录患...     

Valgus osteotomy combined with intramedullary nail for Shepherd’s crook deformity in fibrous dysplasia: 14 femurs with a minimum of 4 years follow-up

Background and objectives  

To investigate the effectiveness of valgus osteotomy combined with intramedullary nail in treatment of Shepherd’s crook deformity of fibrous dysplasia.     

Femoral fractures in osteodystrophy deformans (Paget's disease). Treatment results

Between 1958 and 1986 21 fractures of the femur in Osteodystrophia deformans (Paget's disease) were treated at the accident hospital Meidling; of these 13 patients were female and 8 of male. The average age was 73.4 years. In two cases the basic illness was known, in 19 cases the fracture was the first manifestation of the illness. In 7 cases a polyostotic manifestation was evident. The fractures were localized as follows: 7 in the proximal, 7 in the middle, 1 in the distal third of the shaft, 5 subtrochanteric, 1 supracondylar. Apart from one subtrochanteric torsion fracture, all were flexure fractures without splinters. 19 fractures were stabilized by an operation: 17 by intramedullary nailing (with cerclage), 1 steep plate, 1 Ender nail. One case was treated by an extension, whereas in one case no operation was performed. The following complications were observed: one fracture of the steep plate, one delayed healing of the fracture after intramedullary nailing. No infections could be observed. In general, healing of the fracture in Osteodystrophia deformans does not seem to be delayed. The different possibilities of intramedullary nailing of the femur - possibly together with osteotomies to eliminate curvature - can be recommended for fractures in Osteodystrophia deformans.     

Prophylactic intramedullary nailing in monostotic fibrous dysplasia

Fibrous dysplasia of bone is an enigma with no known cure. Treatment currently consists of curettage and bone-grafting in an attempt to eradicate the lesion and to prevent progressive deformity. This study presents the results of prophylactic intramedullary nailing in 10 patients with monostotic fibrous dysplasia, pain increasing with movement, and scintigraphically established activity. Ten patients with monostotic fibrous dysplasia in their upper or lower extremities treated between 2001 and 2003 were included in the study. Seven patients were male and 3 were female; their mean age was 26.9 years. The mean duration of follow-up was 33.5 months. Closed intramedullary nail without reaming was used in all cases. Bone grafting was not performed. Patients were allowed full weight bearing on the affected extremities on the second postoperative day. Mean VAS for functional pain was 5.33 +/- 0.65 preoperatively and 2.26 +/- 0.57 at final follow-up (p < 0.05). Radiographs showed no changes in lesion size, and the intramedullary fixation appeared to be stable. Prophylactic intramedullary nailing appeared to be beneficial in monostotic fibrous dysplasia with scintigraphically proven activity and functional pain. It also avoids problems that may occur following pathological fracture.     

Flexible intramedullary nailing for stabilization of displaced midshaft clavicle fractures: technique and results in 87 patients

Background Elastic, stable intramedullary nailing (ESIN) with titanium nails is a promising minimally treatment for displaced midclavicular fractures, which may be an alternative to plate fixation (ORIF) or even nonoperative treatment. We describe the surgical technique and outcome in 87 patients.

Methods The nail was inserted at the medial inferior end of the clavicle in 83 patients and in the acromial end in 12 patients. An open fracture reduction via an additional small incision was necessary in 53 patients and closed manoeuvre was successful in 42. Implant removal was performed in 82 patients.

Results The functional status of 87 patients after 13 months reached 6.8 (0-43) points on the DASH score and 81 (46-100) points on the self-reported Constant score. The fracture healed in correct anatomical axis in 80 of 87 patients, 2 cases ended in a nonunion. Implant migration of the nail occurred in 4 patients, who required early implant removal. Repeated nailing was necessary in 2 patients in whom the nail missed the lateral medullar canal, and plate fixation was necessary in 2 other patients who had secondary dislocation after early nail removal.

Interpretation Flexible intramedullary nailing, a minimally invasive technique for stabilization of displaced midshaft clavicle fractures, has minor risks and complications.     

Proximal femoral radiographic changes after lateral transtrochanteric intramedullary nail placement in children

BACKGROUND: Recent reports have described osteonecrosis of the femoral head after intramedullary nailing of the femur through the piriformis fossa in children. Other reports have raised concerns about the development of femoral neck narrowing and valgus deformity of the proximal part of the femur after intramedullary nailing through the tip of the greater trochanter. We evaluated the radiographic changes in the proximal part of the femur following intramedullary nailing through the lateral trochanteric area at a minimum of two years postoperatively in twenty-five affected extremities. The mean age of the patients at the time of the index procedure was ten years and six months. METHODS: A retrospective radiographic review was performed to look for proximal femoral changes. Specifically, the radiographs were examined for evidence of osteonecrosis. The articulotrochanteric distance, femoral neck diameter, and neck-shaft angle were measured on the initial and final radiographs. RESULTS: No patient had evidence of osteonecrosis of the femoral head. The articulotrochanteric distance decreased by a mean of 0.4 mm, the femoral neck diameter increased by a mean of 4.9 mm, and the neck-shaft angle decreased by a mean of 1.4 degrees. Compared with a group of seventeen patients with adequate initial and final radiographs of the contralateral side, the final mean articulotrochanteric distance was 4.5 mm less on the involved side than on the uninvolved side, the mean femoral neck diameter was 0.7 mm less on the involved side than on the uninvolved side, and the mean neck-shaft angle was 3.2 degrees less on the involved side than on the uninvolved side. No patient had development of clinically important femoral neck narrowing or valgus deformity. Statistically, the likelihood that these data represent a group with a mean 3-mm increase in the articulotrochanteric distance is <1%. The likelihood that these data represent a group with a mean 3.2-mm decrease in the ultimate femoral neck diameter is <1%. The likelihood that these data represent a group with a mean 5 degrees increase in the neck-shaft angle is <1%. CONCLUSIONS: Lateral transtrochanteric intramedullary nailing in children who are nine years of age or older does not produce clinically important femoral neck valgus deformity or narrowing, and we did not observe osteonecrosis of the femoral head after this procedure.     

Polyostotic fibrous dysplasia with acromegaly (Albright's syndrome). A case report.

The association of polyostotic fibrous dysplasia and acromegaly, presenting with gross deformity of the skull and fractures of the left femur and right humerus, is described in a 42-year-old Black woman. Possible causative factors are discussed.     

截骨矫形钢板固定治疗股骨近段纤维结构不良伴髋内翻畸形

目的 探讨股骨转子下截骨矫形、动力髋或髁螺钉系统固定治疗股骨近段纤维结构不良(fibrous dysplasia,FD)伴髋内翻畸形的临床疗效.方法 回顾性分析2001年4月至2010年5月收治的26例股骨FD伴髋内翻畸形患者的临床资料,男9例,女17例;年龄10～53岁,中位年龄19岁.单骨型14例,多骨型12例.病灶长度为多骨型9～36 cm(平均19 cm),单骨型7～15 cm(平均9 cm);17例合并病理性骨折;术前颈干角65°～110°(平均92°),患肢短缩1.5～4.5cm(平均2.8 cm).21例病灶累及股骨颈者采用髋螺钉固定,5例未累及股骨颈者采用髁螺钉固定.结果 手术时间80～170 min(平均120min),出血量280～1650ml(平均960ml).术后颈干角为119°～140°(平均127°);患肢平均延长2.3 cm(1.5～3.6 cm).随访9～118个月(平均39个月),截骨面均愈合.除1例颈干角从术后126°减小到术后56个月的115°,其余患者无髋内翻畸形复发,无内固定断裂或松动.1例股骨颈变短、髋螺钉沿滑槽滑动,但螺钉未切割股骨头;1例术后7年外伤后钢板远端处股骨干骨折.根据Guille的功能评价标准,24例满意、2例不满意.结论 转子下截骨矫形、动力髋或髁螺钉系统内固定能有效地纠正股骨上段FD伴髋内翻畸形,改善患肢功能.

Abstract：

Objective To investigate the clinical effect of subtrochanteric osteotomy plus dynamic hip/condyle screw (DHS/DCS)fixation to treat the fibrous dysplasia of the proximal femur with coxa vara.Methods Twenty-six clinical cases of femoral fibrous dysplasia with coxa vara were retrospectively analysed from April 2001 to May 2010.There were 9 males and 17 females,with a median age of 19(10 to 53).Forteen patients presented with monostotic disease,and 12 with polyostotic disease.The length of the lesion for polyostotic disease was from 9 cm to 36 cm,while for monostotic disease was from 7 cm to 15 cm.Seventeen cases were merged with pathologic fracture.The neck shaft angles were from 65 to 110 degree preoperation.The shortage of limbs was from 1.5 cm to 4.5 cm.Twenty-one patients involved in femoral neck were fixed with DHS and the other 5 cases with DCS.Results The duration of operation was from 80 to 170 min.The amout of bleeding was from 280 to 1650 ml with the average of 960 ml.The average postoperative neck shaft angles were 127 degree(119 to 140).The shortened limbs were extended 2.3 cm for average (1.5 to 3.6 cm).The follow-up time was from 9 to 118 months with the average of 39 months.All patients with osteotomy were healed.The neck shaft angle of 1 case decreased from post-operative 126°to 115°56months post-operatively,no coxa adducta recurrented and all internal fixations were in position.Hip screw backed out through the barrel in one case with the shortage of femoral neck.One case had femoral fracture after an injury.According to Guille function standard,24 cases were regarded as satisfied and 2 cases as unsatisfied.Conclusion Subtroehanteric osteotomy plus DHS/DCS fixation can effectively correct the fibrous dysplasia of the proximal femur with coxa vara,and significantly improve the function.     

Combination of femoral fracture treatment and corrective osteotomy in a child with osteogenesis imperfecta

The main feature of osteogenesis imperfecta is an excessive fragility and deformability of the bones owing to reduced mass and bone quality. This leads to angular deformity and frequent fractures. These fractures usually heal rapidly, and conservative treatment is the norm. In displaced and unstable fractures, elastic intramedullary nailing is a treatment option. We report a case of a 3-year-old child with osteogenesis imperfecta type I who suffered an undisplaced femoral shaft fracture in the presence of a preexisting 32° femoral antecurvation. This deformity greatly increases the risk of a refracture due to the pathological induction of stress risers. Therefore, fracture treatment by unreamed elastic intramedullary nailing was combined with simultaneous correction osteotomy, resulting in anatomic alignment and uncomplicated fracture healing. The single-stage surgical stabilization performed allowed rapid mobilization along with a decreased likelihood of refracture.